Objective: To investigate the value of plasma cells for diagnosing lymph node diseases. Methods: Common lymphadenopathy (except plasma cell neoplasms) diagnosed from September 2012 to August 2022 were selected from the pathological records of Changhai Hospital, Shanghai, China. Morphological and immunohistochemical features were analyzed to examine the infiltration pattern, clonality, and IgG and IgG4 expression of plasma cells in these lymphadenopathies, and to summarize the differential diagnoses of plasma cell infiltration in common lymphadenopathies. Results: A total of 236 cases of lymphadenopathies with various degrees of plasma cell infiltration were included in the study. There were 58 cases of Castleman's disease, 55 cases of IgG4-related lymphadenopathy, 14 cases of syphilitic lymphadenitis, 2 cases of rheumatoid lymphadenitis, 18 cases of Rosai-Dorfman disease, 23 cases of Kimura's disease, 13 cases of dermal lymphadenitis and 53 cases of angioimmunoblastic T-cell lymphoma (AITL). The main features of these lymphadenopathies were lymph node enlargement with various degrees of plasm cell infiltration. A panel of immunohistochemical antibodies were used to examine the distribution of plasma cells and the expression of IgG and IgG4. The presence of lymph node architecture could help determine benign and malignant lesions. The preliminary classification of these lymphadenopathies was based on the infiltration features of plasma cells. The evaluation of IgG and IgG4 as a routine means could exclude the lymph nodes involvement of IgG4-related dieases (IgG4-RD), and whether it was accompanied by autoimmune diseases or multiple-organ diseases, which were of critical evidence for the differential diagnosis. For common lesions of lymphadenopathies, such as Castleman's disease, Kimura's disease, Rosai-Dorfman's disease and dermal lymphadenitis, the expression ratio of IgG4/IgG (>40%) as detected using immunhistochemistry and serum IgG4 levels should be considered as a standard for the possibility of IgG4-RD. The differential diagnosis of multicentric Castleman's diseases and IgG4-RD should be also considered. Conclusions: Infiltration of plasma cells and IgG4-positive plasma cells may be detected in some types of lymphadenopathies and lymphomas in clinicopathological daily practice, but not all of them are related to IgG4-RD. It should be emphasized that the characteristics of plasma cell infiltration and the ratio of IgG4/IgG (>40%) should be considered for further differential diagnosis and avoiding misclassification of lymphadenopathies.
Humans ; Castleman Disease/pathology* ; Plasma Cells/pathology* ; Immunoglobulin G4-Related Disease ; China ; Lymphadenopathy/pathology* ; Inflammation/pathology* ; Lymph Nodes/pathology* ; Diagnosis, Differential ; Lymphadenitis/pathology* ; Immunoglobulin G/metabolism*

Objective: To investigate the clinical features, pathological phenotype, treatment and prognosis of Castleman's disease in children. Methods: Clinical data of 15 children diagnosed with Castleman's disease in Henan Provincial People's Hospital and the First Affiliated Hospital of Zhengzhou University from May 2010 to October 2019 were analyzed retrospectively. The clinical characteristics, laboratory examination and histopathological data were analyzed. Results: Among the 15 Castleman's disease patients, 12 were males and 3 females. The age of first visit was 12 (10, 15) years. The time from mass discovery to pathologic diagnosis was 9.0 (2.0, 13.0) months. The majority of patients were unicentric (13 cases), and the histopathological type was hyaline vascular (11 cases). Unicentric lesions were most common in the neck (11 cases), all 13 patients received complete surgical resection of the lesions, the follow-up time was 20.0 (13.5, 50.5) months, and the prognosis was good. Two cases were multicentric type, the pathological types were mixed variant, meeting the criteria of idiopathic Castleman's disease, the two children underwent partial surgical resection, one was treated with rituximab and prednisone and the other was treated with thalidomide and prednisone. The follow-up time was 32 months and 10 month, both of them had good prognosis. Conclusions: Most cases of Castleman's disease in children are diagnosed late, and the unicentric type is dominant. The most common pathological type is hyaline vascular, which is characterized by painless lymphadenopathy, while multicentric type has systemic symptoms and both of them have a good overall prognosis.
Castleman Disease/therapy* ; Female ; Humans ; Male ; Neck/pathology* ; Prognosis ; Retrospective Studies ; Rituximab

Paraneoplastic pemphigus is a rare autoimmune bullous dermatosis, which is caused by potential neoplasm, especially the Castleman's disease. Castleman's disease associated with paraneoplastic pemphigus is misdiagnosed frequently and easily in clinical practices. Furthermore, it is reported that the mortality rate for this disease is very high. Bronchiolitis obliterans is the most common complication and the most important cause of death. There was a female patient presenting recalcitrant mucocutaneous erosions, ulcers and scattered erythemas in the body. The patient was diagnosed and treated for pemphigus vulgaris with little success in Xiangya Hospital, Central South University in January 2015. Further investigations confirmed the diagnosis of paraneoplastic pemphigus with retroperitoneal tumor. Subsequently, the patient was treated with tumor resection in combination with intravenous immunoglobulin and corticosteroids. The pathology revealed that it was the Castleman's disease. Her mucocutaneous performance recovered obviously and the bronchiolitis obliteran did not appear in the follow-up. Castleman's disease associated with paraneoplastic pemphigus should be considered when mucosal and skin lesions showing no improvement under corticosteroids. Early and complete removal of the tumor together with immunotherapy could be beneficial to the patient's prognosis.
Adrenal Cortex Hormones ; therapeutic use ; Castleman Disease ; complications ; therapy ; Female ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Paraneoplastic Syndromes ; complications ; therapy ; Pemphigus ; complications ; therapy ; Retroperitoneal Space ; pathology

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Castleman Disease ; immunology ; pathology ; Cyclophosphamide ; therapeutic use ; Diagnostic Errors ; Doxorubicin ; therapeutic use ; Humans ; Immunoglobulin G ; blood ; Kidney ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Lymphatic Diseases ; drug therapy ; immunology ; pathology ; surgery ; Male ; Middle Aged ; Nephrectomy ; Pancreas ; Plasma Cells ; immunology ; pathology ; Prednisone ; therapeutic use ; Submandibular Gland ; Vincristine ; therapeutic use

OBJECTIVETo analyze the CT and MRI findings and pathological characteristics of retroperitoneal localized Castleman's disease and discuss the diagnostic and differential value of CT and MRI.

METHODSCT, MRI and clinical data of retroperitoneal localized Castleman's disease, proved by surgery and pathology, of 13 patients were reviewed. Among them, all the 13 cases received CT, and 4 cases received MRI examination.

RESULTSAmong the 13 cases, 12 cases were of hyaline vascular type, and one was of mixed type. All foci were single mass and most foci located in the periphery of the kidney. The maximum diameter was 4.2 cm to 8.6 cm and the mean diameter of all tumors was 5.9 cm. The outline of most tumors was clear and kidney-shaped. On unenhanced CT, 10 cases showed low density and 3 cases showed isodensity. On plain MRI, four cases showed iso-signal on T1WI, three cases showed slightly high signal on T2WI and one showed moderately high signal. The CT and MRI findings were similar on contrast enhanced scan. In arterial phase, 10 cases showed moderate enhancement, 2 cases showed significant enhancement and one case showed moderate enhancement. Five cases showed rather homogeneous enhancement and 8 cases showed heterogeneous enhancement.In venous phase, all the 13 tumors showed continuous enhancement. Eight cases showing heterogeneous enhancement in arterial phase showed expanded range, and among them two cases showed tending to be homogeneous enhancement. Six cases showed areas of low density without enhancement, and 9 cases were accompanied with single or multi-satellite punctuate or striped foci around the tumors.

CONCLUSIONDynamic contrast-enhanced CT and MRI combined with MR T2WI fat-suppression are of great importance in the diagnosis and identification of retroperitoneal localized Castleman's disease.

Adult ; Castleman Disease ; diagnostic imaging ; pathology ; surgery ; Female ; Humans ; Image Enhancement ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Radiographic Image Enhancement ; Retroperitoneal Space ; diagnostic imaging ; Retrospective Studies ; Tomography, X-Ray Computed

OBJECTIVETo evaluate the expression of interleukin-6 (IL-6) and its clinicopathological significance in Castleman's disease (CD).

METHODSClinical data and paraffin blocks of 92 CD patients and 20 cases of lymph node reactive hyperplasia (LRH) as a control group were collected from department of pathology of Peking University Health Science Center. The expression of IL-6 was detected by using immunohistochemical method.

RESULTSThe 92 patients were composed of 42 multicentric variant (MCDs) and 50 unicentric variant (UCDs) clinically, and 30 hyaline-vascular variant (HV-CDs) and 62 plasma cell variant (PC-CDs) morphologically. None of them was positive for HIV tests. There were 56 males and 36 females, and their ages ranged from 4 years to 90 years with the median 41 years. IL-6 was expressed in 77 (83.7%) of 92 CD cases and 1 (5.0%) of 20 LRH cases. The expression rate of IL-6 was 90.5% in MCDs, 78.0% in UCDs, 93.6% in PC-CDs and 63.4% in HV-CDs, respectively. PC-CD cases showed a significantly higher expression rate of IL-6 than HV-CD cases (P = 0.001). All cases with positive IL-6 expression in plasmacytes were PC-CDs, showing obviously higher expression in MCDs than that in UCDs (P = 0.003). Compared with HV-CD cases, much more PC-CD cases showed IL-6 positivity in endothelial cells (P = 0.008). However, IL-6 was rarely expressed by both FDCs and macrophages, with only 3.3% and 10.9% positive cases, respectively. There are 53.2% (41/77) of the IL-6 positive cases and 20.0% (3/15) of IL-6 negative cases suffered from systemic symptoms, showing a significant difference between the two groups (P = 0.018). Cases with IL-6 expression in plasmacytes and macrophages were more likely to suffer from systemic symptoms, especially B type symptoms (P < 0.05).

CONCLUSIONThere is a high expression rate of IL-6 in CD, which is different from LRH. The expression of IL-6 has close relationship with CD subtypes and the presence of systemic symptoms. In all, the evaluation of interleukin-6 is of great value to guide the diagnosis and therapy of CD.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Castleman Disease ; metabolism ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Interleukin-6 ; metabolism ; Male ; Middle Aged ; Young Adult

Aged ; Castleman Disease ; immunology ; pathology ; Diagnosis, Differential ; Humans ; Immunoglobulin G ; metabolism ; Lymphatic Diseases ; immunology ; pathology ; surgery ; Lymphoma ; pathology ; Male ; Plasma Cells ; immunology ; Pseudolymphoma ; immunology ; pathology

To evaluated the multi-detector CT (MDCT) and magnetic resonance imaging (MRI) features of localized Castleman's disease (CD), we retrospectively analyzed the clinical data, MDCT and MRI findings of 13 patients with CD proved pathologically. All patients underwent plain MDCT scan, 11 underwent enhanced CT, and 2 MRI. 14 lesions were detected in the 13 patients, and all of them were hyaline-vascular type (HV-CD) histopathologically. On plain MDCT scans, all lesions were homogeneously attenuated soft tissue mass; intra-tumoral calcification with punctate and "arborizing" patterns was detected in the center of 2 lesions each. Of the patients with enhanced MDCT, all lesions showed obvious enhancement homogeneously except two lesions with central stellate and 1 lesion with dotted low attenuation. Tortuous vessels could be revealed at the periphery of 8 lesions. Of the 2 patients with MRI, the lesions showed slightly hyper-intensity on T1WI, hyper-intensity on T2WI and marked homogenous enhancement. In addition, one of them showed signal void appearance in the center on unenhanced MRI and large supplying artery with tortuous vessels at the periphery on enhanced MRI. In a word, Localized HV-CD usually demonstrated as soft tissue mass with obvious enhancement on MDCT and MRI. Central stellate area of low attenuation and calcification with punctate or "arborizing" pattern may also be present.
Adolescent ; Adult ; Castleman Disease ; diagnosis ; diagnostic imaging ; pathology ; Female ; Humans ; Image Enhancement ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multidetector Computed Tomography ; Retrospective Studies ; Young Adult

OBJECTIVETo investigate the clinical characteristics and treatment of localized Castleman's disease (CD), and review the literatures to improve the diagnosis and management of this disease.

METHODSThe clinical symptoms, histopathology, CT, MRI findings and results of surgery in 20 patients with localized CD were evaluated retrospectively.

RESULTSThe average age of the patients was 37.7 years. The lesions were located in the retroperitoneal space (9 cases), mediastinum (7 cases), pelvic cavity (1 case), neck (1 case), upper arm (1 case), and axillary (1 case). All patients underwent surgical resection, including 9 cases for retroperitoneal resection (6 cases had open operation and 3 cases laparoscopic resection) and 7 cases for mediastinal resection (open operation in 5 cases and thoracoscopic resection in 2 cases). The Castleman's disease was confirmed by histopathology. There were hyaline vascular type of CD in 17 cases, plasma cell type of CD in 1 case, and mixed cellularity type of CD in 2 cases. The duration of follow-up ranged from 12 to 165 months for 16 cases. Among them 15 patients were alive without recurrence, and 1 case had recurrence in the primary site at 47 months after the operation.

CONCLUSIONSPatients with Castleman's disease have no typical clinical symptoms and have normal laboratory results. The majority of patients are of hyaline vascular type of the disease. Imaging examination is helpful to diagnosis, and the final diagnosis depends on pathologic examination. Complete surgical resection of the tumor is the best treatment for localized Castleman's disease.

Adult ; Castleman Disease ; diagnosis ; diagnostic imaging ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Mediastinum ; Middle Aged ; Recurrence ; Retroperitoneal Space ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome ; Young Adult

ADP-ribosyl Cyclase 1 ; metabolism ; Adult ; Brain Neoplasms ; metabolism ; pathology ; surgery ; CD79 Antigens ; metabolism ; Castleman Disease ; metabolism ; pathology ; Diagnosis, Differential ; Humans ; Leukocyte Common Antigens ; metabolism ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; surgery ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Melanoma ; metabolism ; pathology ; Parietal Lobe ; Plasma Cells ; metabolism ; pathology